Plastic Surgery Appointment...

Lifeisgood · #1 12-14-2007, 08:42 PM

So, for all you PRS folks (and other underdeveloped jaw folks) I have some questions. First, Harlie doesn't have PRS (I think you have to have a cleft palate, too, for that, right)? She doesn't have a cleft palate. Just the underdeveloped lower jaw. She's missing several bones as well. Anyway, we have seen 4 ENTs now. I have asked each one about using a PMV. To me, it just doesn't make sense that it would be safe for her. It is an upper airway obstruction. They have all said that she will move her tongue out of the way. So far, the PMV has not gone well. She cannot exhale through her mouth.

So, on Tuesday we saw her plastic surgeon about jaw reconstruction surgery. He said that her mandible is so small and that her tongue base is basically in her airway. He said she is incapable of moving it out the way. He does not see how it would be possible for her to exhale without the trach (or swallow for that matter).

So, what have you guys experienced on this subject? I know there are different degrees of underdevelopment. So far, I've been told that Harlie's is very severe. Do your kids tolerate the PMV and what are your plans for reconstruction surgery?

Our plan is to have another CT scan done in Feb/Mar and then surgery in MAY! YAHOO! He said that jaw distraction won't work for her. He is going to go in and cut her jaw, and insert bone taken from her skull to extend her jaw. He will place it where he wants it and then wire it shut like that for 8 weeks. So, right after surgery, she will have a chin! Oh, they are going to replace the bone taken from her skull with cadaver bone. Weird. Anyway, has anyone else done this?

Any info on this will be very much appreciated. Thanks!

-Christy

sixpackmom · #2 12-14-2007, 08:54 PM

Christy my daughter Meagan has PRS. She did have a cleft palete, but you can have prs without having a cleft palete. She had I guess what would be considered moderate prs, her tongue did obstruct her airway, but she was able to have the madibular distraction. She could not tolerate a pmv either, it would make her cough and she could shoot it across the room. After her distraction surgery she was able to start tolerating it for short periods of time. She had her hardware placed in April and we turned the screws twice daily for 2 weeks, she had it removed in July and then was able to be decanned in September.
Does she have any other medical problems besides the small jaw?
Shelly

Sugarbabies · #3 12-14-2007, 10:55 PM

Hi, I have 3 kids with PRS. My oldest outgrew hers naturally and I was just told yesterday by the orthodontist that her overbite is only 10% or so. Regular braces will correct her bite, just like any other kid.

By the pictures I see of Harley, her case is more severe than my children. They did begin with an almost non-existent chin, but in the last year the chin has almost caught up with the rest of their face. (Yeah, that's a weird statement, isn't it?)

There is a forum like this for PRS kids. There are several parents who have gone through the mandibular distraction and could give you a realistic idea of what you and Harley are in for. Pierrerobin.org I think.

About the PMV.... It could definitely be the tongue in the way. Of my indentical twins, I have one that can tolerate the PMV (but prefers to plug his trach with his finger) and one that cannot tolerate it at all because he has granuloma tissue above his trach, which blocks air attempting to escape out his mouth. (This will be repaired at the same time as their cleft palates are repaired in March.)

I am praying that the trachs will come out 2-3 months afterward.

Good luck with Harley! She's beautiful!

RobiL · #4 12-15-2007, 09:35 AM

Ok Yeah i'm an adult. I dont' have PRS but my tongue is blocking my airway. They are talking about me having the jaw distraction surgery. Plastics is all gung ho about it. ENT was gungho at first. Now them and I are thinking about not doing it. They mentioned chin first and if that didnt' work then do bottom or top or both. The plastics guy I saw has done tons with kids but not many on adults. So it's a 50/50 with me. Would you parents recomend it?

Trachs aren't new to me. I've either had one in or been around them all my life.

Robin

faywrayy · #5 12-15-2007, 01:40 PM

Kate has PRS with the cleft palate, but her jaw has grown out significantly since she was a newborn. Here's a pic of her on day of life 2:

You can see she looks close to typical now, so we don't anticipate the distraction for her. As of her bronch on 12/6, her tongue lolls back a bit still, but the ENT still anticipates decann within the next year or so...we hope!

SHe was tried on the PMV maybe 3 weeks post trach placement, and it was miserable. She wasn't able to do it until she was maybe 6 or 7 mos. Now, she's fine, no distress, so I guess that backs up the theory that the tongue blocking makes them unable to use it.

faywrayy · #6 12-15-2007, 02:09 PM

Here's a link to a PRS message board:

http://members3.boardhost.com/PierreRobinNet/

and there's another on Yahoo Groups:

http://health.groups.yahoo.com/group...n/messages?o=1